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PRIMARY ANGLE CLOSURE GLAUCOMA

Wallace L.M. Alward, M.D.Frederick C. Blodi Chair

Department of Ophthalmology

University of Iowa Carver College of Medicine

QUESTION 1:

WHICH OF THE FOLLOWING IS A MAJOR RISK FACTOR FOR

DEVELOPING PUPILLARY BLOCK ANGLE CLOSURE

GLAUCOMA?

A. Male gender

B. Hyperopia

C. Caucasian race

D. High body mass index

QUESTION 2:

WHICH OF THE FOLLOWING IS THE MOST DEFINITIVE

TREATMENT FOR PUPILLARY BLOCK ANGLE CLOSURE?

A. Phacoemulsification

B. Iridoplasty

C. Chronic pilocarpine

D. Baerveldt seton

QUESTION 3:

IN NANOPHTHALMOS WHAT STRUCTURAL ABNORMALITY

PLACES THE PATIENT AT RISK FOR POST-OPERATIVE

COMPLICATION??

A. Small pupil

B. Liquid vitreous

C. Thin and floppy iris

D. Thick sclera

LECTURE OBJECTIVES

To recognize primary pupillary block angle closure as a common form of glaucoma

To recognize primary pupillary block angle closure as a leading cause of blindness – especially in Asia

To recognize the major risk factors: hyperopia, female gender, older age, family history

Pupillary block treated with iridotomy

Phacoemulsification may be a more definitive therapy

Warn family members of their increased risk

INTRODUCTION

A form of glaucoma caused by the iris coming forward to lie over the trabecular meshwork

INTRODUCTION

A form of glaucoma caused by the iris coming forward to lie over the trabecular meshwork

This can cause an abrupt elevation of the intraocular pressure (acute angle closure)

INTRODUCTION

A form of glaucoma caused by the iris coming forward to lie over the trabecular meshwork

This can cause an abrupt elevation of the intraocular pressure (acute angle closure)

Can also cause intermittent or chronic pressure elevation

PATHOPHYSIOLOGY

A relative seal forms between the iris and the lens, trapping aqueous behind the iris, driving it forward

EPIDEMIOLOGY

Race

EPIDEMIOLOGY

Race

• much more prevalent in Asians– in China it causes 91% of bilateral blindness

– Asian angle closure responds less well to LPI

EPIDEMIOLOGY

Hyperopia

• small eye

• shallow AC (<2.5mm)

EPIDEMIOLOGY

Hyperopia

• small eye

• shallow AC (<2.5mm)

Older age

• the lens thickens and pupil becomes smaller with age

EPIDEMIOLOGY

Hyperopia

• small eye

• shallow AC (<2.5mm)

Older age

• the lens thickens and pupil becomes smaller with age

Women (2-4x risk)

EPIDEMIOLOGY

Hyperopia

• small eye

• shallow AC (<2.5mm)

Older age

• the lens thickens and pupil becomes smaller with age

Women (2-4x risk)

Family history

Kavitha S, Zebardast N, Palaniswamy K et al. Ophthalmology 2014;122:2091-2097

The first-degree relatives of patients with angle closure are at high risk for developing the disease (on the order of 35%) and should be screened.

FAMILY RISK

PRECIPITATING FACTORS FOR ACG

Dim illumination

Emotional stress

Mydriasis

• this is where all the warning labels comes from– anticholinergics, antihistamine, antidepressant,adrenergics, CNS stimulants,

bronchodilators

• not during full mydriasis

Intense miosis

• cholinergics

THREE FORMS OF PRIMARY PUPILLARY BLOCK ANGLE

CLOSURE

Acute

Intermittent (sub acute)

Chronic

THREE FORMS OF PRIMARY PUPILLARY BLOCK ANGLE

CLOSURE

Acute

Intermittent (sub acute)

Chronic

SYMPTOMS - ACUTE

Eye pain (often severe)

Headache

Blurred vision

Colored haloes around lights

Nausea & vomiting

CASE

49 yo man presents with acute loss of vision and pain OD

4 day h/o of intermittent R-sided headache and pain OD with blurry vision

Gradually got worse

CASE

On day of presentation, pain is constant and headache severe, feeling of nausea

No history of trauma

CASE

CASE

• What two questions should you ask yourself when you see a patient like this?

CASE

• What is the refractive

error?

CASE

• What does the other

angle look like?

CASE

OD OS

Va CF 20/20

IOP 50 mmHg 10 mmHg

Refraction

(SE)

+4.25 D +4.75 D

SIGNS - ACUTE

Injection

Cloudy cornea

Mid-dilated pupil, fixed

Very high IOP

Iris bombé

IRIS BOMBÉ

IRIS BOMBÉ ON GONIOSCOPY

SIGNS - ACUTE

If the attack breaks spontaneously the patient may have flare, cell and transient hypotony

SEQUELA - ACUTE

Sector iris atrophy

Glaukomflecken

Peripheral anterior synechiae

Pigment deposit on iris and cornea

Disc hyperemia

Disc pallor and cupping

SECTOR IRIS ATROPHY / SPIRALING

IRIS ATROPHY

GLAUKOMFLECKEN

GLAUKOMFLECKEN

THREE FORMS OF PRIMARY PUPILLARY BLOCK ANGLE

CLOSURE

Acute

Intermittent (sub acute)

Chronic

SYMPTOMS – INTERMITTENT (SUB ACUTE)

Intermittent eye pain and/or headache

May be associated blurred vision

Sometimes colored haloes around lights

CASE

• 48 yo female radiologist

• Intractable headaches for years

CASE

• 48 yo female radiologist

• Intractable headaches for years

• Several neurological work-ups

– MRI x 2

– CT

– lumbar Puncture

CASE

Had gonioscopy in the Neuro-Ophthalmology clinic

Found to have critically narrow angles

Iridotomy was curative (with nine year follow-up)

SIGNS - INTEMITTENT

Often only occludable angles on gonioscopy

THREE FORMS OF PRIMARY PUPILLARY BLOCK ANGLE

CLOSURE

Acute

Intermittent (sub acute)

Chronic

SYMPTOMS - CHRONIC

Like primary open angle glaucoma there are no symptoms until late

Gradual decrease in peripheral and night vision

Late loss of central vision

SIGNS - CHRONIC

Elevated intraocular pressure

± Optic nerve cupping

± Visual field loss

Narrow angles on gonioscopy

• often with extensive peripheral anterior synechiae

DIFFERENTIAL DIAGNOSIS

Plateau iris

Phacomorphic

Nanophthalmos

Aqueous misdirection

Ciliary body swelling or inflammation following PRP, SB, drugs (topiramate, cold meds)

Tumors

DIFFERENTIAL DIAGNOSIS

Plateau iris (often has pupillary block)

Phacomorphic (has pupillary block)

Nanophthalmos (has pupillary block)

Aqueous misdirection

Ciliary body swelling or inflammation following PRP, SB, drugs (topiramate, cold meds)

Tumors

TREATMENT

Laser iridotomy or (rarely) surgical iridectomy

• to break the pupillary block

LASER PERIPHERAL IRIDOTOMY

LASER PERIPHERAL IRIDOTOMY

TREATMENT

Laser iridotomy or (rarely) surgical iridectomy

• to break the pupillary block

• after the iridotomy the angles should be deeper, but are rarely deep

TREATMENT OF AACG

Medications

• all of the drops

• hold cholinergics while IOP until IOP <35 or so

• systemic CAI, hyperosmotics

TREATMENT OF AACG

Medications

• all of the drops

• hold cholinergics while IOP until IOP <35 or so

• systemic CAI, hyperosmotics

Mechanical

• corneal indentation

TREATMENT: CORNEAL INDENTATION

TREATMENT OF AACG

Medications

• all of the drops

• hold cholinergics while IOP until IOP <35 or so

• systemic CAI, hyperosmotics

Mechanical

• corneal indentation

Surgical

• iridotomy / surgical iridectomy

• iridoplasty if the view is inadequate

SURGICAL TREATMENT

Surgical iridectomy

• rarely done

• uncooperative for Laser PI

• if following attack, consider trabeculectomy at the same

time

SURGICAL TREATMENT

Goniosynechiolysis

• To break PAS

• Usually with cataract surgery

• Only works for “fresh” PAS (<12 months)

SURGICAL TREATMENT

Trabeculectomy

• these patients are at increased risk for aqueous misdirection

• use long-term atropine post-operatively

MANAGEMENT OF AACG

Don’t forget the other eye

• Untreated fellow eye has 40-80% chance of having AAC in 5-10 yrs.

• Some risk to the fellow eye during the acute attack because of the sympathetic stimulation

PROPHYLACTIC IRIDOTOMY INDICATIONS

Elevated IOP with appositional closure

≥ 180 degrees of apposition

Narrow angle with PAS

Increased segmental pigmentation from recurrent contact

History of AACG in fellow eye

Iridotomy in the fellow eye

Do serial gonioscopy to monitor angle even after the LPI

PROPHYLACTIC IRIDOTOMY

If you worry enough that the patient will have an attack of pupillary block angle closure that you warn them about symptoms and tell them to avoid cold medications and dark restaurants –you should do an LPI.

PROPHYLACTIC IRIDOTOMY INDICATIONS

“MIXED MECHANISM” GLAUCOMA

A patient with narrow angles who continues to have high IOP despite angles deepening after iridotomy

“MIXED MECHANISM” GLAUCOMA

A patient with narrow angles who continues to have high IOP despite angles deepening after iridotomy

A patient who starts as POAG, but whose

angles narrow (lens growth, exfoliation,

cholinergics)

THE EAGLE STUDY

There is evidence that clear lens extraction may be superior to iridotomy – especially in Asian populations.

Azuara-Blanco A et.al. Lancet 2016: 388; 1389-1307

THE EAGLE STUDY

Randomized comparison of iridotomy vs. clear lens extraction in patients with primary angle closure and primary angle closure glaucoma

30 centers in five countries

419 patients (>50 y.o., no symptomatic cataracts)

~30% of Chinese origin

Azuara-Blanco A et.al. Lancet 2016: 388; 1389-1307

THE EAGLE STUDY

Those assigned to phacoemulsification had:

better quality of life indices (despite no

symptomatic lens opacities)

lower IOP (by 1 mmHg)

far fewer medications (21% vs 61%)

Azuara-Blanco A et.al. Lancet 2016: 388; 1389-1307

THE EAGLE STUDY

“Clear-lens extraction showed greater efficacy and was more cost-effective than laser peripheral iridotomy, and should be considered as an option for first-line treatment.”

Azuara-Blanco A et.al. Lancet 2016: 388; 1389-1307

NANOPHTHALMOS

Small eye that is structurally mostly normal – unlike microophthalmos

NANOPHTHALMOS

Short eye <~20 mm, high hyperopia

NANOPHTHALMOS

Short eye <~20 mm, high hyperopia

Angle closure at early age

NANOPHTHALMOS

Short eye <~20 mm, high hyperopia

Angle closure at early age

Thick and impermeable sclera

NANOPHTHALMOS

21.04 mm AEL

2.4 mm thick sclera

51.5 D calculated IOL

Courtesy of A. Tim Johnson, MD, PhD - the University of Iowa.

NANOPHTHALMOS

Short eye <~20 mm, high hyperopia

Angle closure at early age

Thick and impermeable sclera

Dominant

NANOPHTHALMOS

Try to avoid intraocular surgery

Early LPI and perhaps iridoplasty

Do scleral windows with intraocular surgery

QUESTION 1:

WHICH OF THE FOLLOWING IS A MAJOR RISK FACTOR FOR

DEVELOPING PUPILLARY BLOCK ANGLE CLOSURE

GLAUCOMA?

A. Male gender

B. Hyperopia

C. Caucasian race

D. High body mass index

QUESTION 2:

WHICH OF THE FOLLOWING IS THE MOST DEFINITIVE

TREATMENT FOR PUPILLARY BLOCK ANGLE CLOSURE?

A. Phacoemulsification

B. Iridoplasty

C. Chronic pilocarpine

D. Baerveldt seton

QUESTION 3:

IN NANOPHTHALMOS WHAT STRUCTURAL ABNORMALITY

PLACES THE PATIENT AT RISK FOR POST-OPERATIVE

COMPLICATION??

A. Small pupil

B. Liquid vitreous

C. Thin and floppy iris

D. Thick sclera

LECTURE OBJECTIVES

To recognize primary pupillary block angle closure as a common form of glaucoma

To recognize primary pupillary block angle closure as a leading cause of blindness – especially in Asia

To recognize the major risk factors: hyperopia, female gender, older age, family history

Pupillary block treated with iridotomy

Phacoemulsification may be a more definitive therapy

Warn family members of their increased risk

THANK YOU

http://curriculum.iowaglaucoma.org/iBook store

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